[A case of lymphogranulomatosis in a patient with type 1 neurofibromatosis]

Neurofibromatosis is a common monogenic disease in man; its incidence is at least 1:3000-1:4000 population. It is inherited in the autosomal dominant mode with high penetrance and variable expression. A patient presenting with type 1 neurofibromatosis since the age of 3 mo who developed lymphogranulomatosis is reported. The patient 1 life prognosis is presently determined by the competing pathology. It is emphasized that neurofibromatosis is associated with different forms of lung lesions and creates a risk of neoplastic (including malignant) growth.